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Pediatric Radiology

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27-12-2023 | Beckwith-Wiedemann Syndrome | Original Article

Reversed cortico-medullary differentiation in the fetal and neonatal kidneys: an indicator of poor prognosis?

Authors: Marie Cassart, Catherine Garel, Tim Ulinski, E. Freddy Avni

Published in: Pediatric Radiology | Issue 2/2024

Abstract

Background

Bilateral reversed cortico-medullary differentiation is rarely observed on fetal or neonatal renal ultrasound and is therefore a diagnostic challenge.

Objective

Our purpose was to widen the differential diagnoses of fetal and neonatal nephropathies introducing reversed cortico-medullary differentiation as a clue either on obstetric US or during follow-up of hyperechoic kidneys in order to improve the management of such rare clinical situations.

Materials and methods

We retrospectively reviewed the US images of 11 patients showing bilateral reversed cortico-medullary differentiation on prenatal examination or in which this pattern developed postnatally in the follow-up of fetal hyperechoic kidneys. For each patient, a precise diagnosis was established either on clinical assessment or, when available, on histological or genetic findings.

Results

Six fetuses displayed bilateral reversed cortico-medullary differentiation on obstetric examination, and the pattern persisted throughout pregnancy. In the five other fetuses, the kidneys appeared initially homogeneously hyperechoic; this evolved into reversed cortico-medullary differentiation during the third trimester in two cases and shortly after birth in three cases. Two pregnancies were terminated because of estimated poor prognosis. In the nine surviving neonates, four died of renal failure in the post-natal period. The clinical evolution was more favorable in the remaining five newborns.

Conclusions

Six different diagnoses were established in patients presenting with a reversed cortico-medullary differentiation renal pattern. This finding was associated with poor outcome in six cases. An acute prenatal diagnosis of reversed cortico-medullary differentiation improves pre- and postnatal work-up and guides counseling and genetic testing.

Graphical abstract

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Title: Reversed cortico-medullary differentiation in the fetal and neonatal kidneys: an indicator of poor prognosis?
Authors: Marie Cassart
Catherine Garel
Tim Ulinski
E. Freddy Avni
Publication date: 27-12-2023
Publisher: Springer Berlin Heidelberg
Keywords: Beckwith-Wiedemann Syndrome
Beckwith-Wiedemann Syndrome
Hypercalcemia
Published in: Pediatric Radiology / Issue 2/2024
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-023-05833-0

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Reversed cortico-medullary differentiation in the fetal and neonatal kidneys: an indicator of poor prognosis?

Abstract

Background

Objective

Materials and methods

Results

Conclusions

Graphical abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Objective

Materials and methods

Results

Conclusions

Graphical abstract

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